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Mutations in MYH9 are associated with hematologic abnormalities, renal dysfunction, and hearing loss. Bony cochlear nerve canal stenosis (CNCS), which is diagnosed on computed tomography (CT) imaging, has been associated with congenital deafness, cochlear nerve aplasia\/hypoplasia, and inner ear malformations. We report two cases of CNCS presenting with profound congenital hearing loss whom we diagnosed with mutations in MYH9 and discuss the genotype-phenotype association and implications for management.<\/jats:p>","DOI":"10.1177\/01455613221135644","type":"journal-article","created":{"date-parts":[[2022,10,25]],"date-time":"2022-10-25T12:46:41Z","timestamp":1666702001000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Heterozygous\n                    <i>MYH9<\/i>\n                    Mutations in 2 Children With Cochlear Nerve Canal Stenosis"],"prefix":"10.1177","volume":"104","author":[{"given":"Wenqi","family":"Liang","sequence":"first","affiliation":[{"name":"Capital Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Line","family":"Wang","sequence":"additional","affiliation":[{"name":"Capital Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wenrui","family":"Zheng","sequence":"additional","affiliation":[{"name":"Capital Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shuguang","family":"Han","sequence":"additional","affiliation":[{"name":"Capital Medical University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9166-0869","authenticated-orcid":false,"given":"Kevin A.","family":"Peng","sequence":"additional","affiliation":[{"name":"House Clinic and House Institute Foundation"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2022,10,25]]},"reference":[{"issue":"4","key":"e_1_3_4_2_2","first-page":"635","article-title":"Imaging findings of cochlear nerve deficiency","volume":"23","author":"Glastonbury CM","year":"2002","unstructured":"Glastonbury CM, Davidson HC, Harnsberger HR, Butler J, Kertesz TR, Shelton C. 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